脊索瘤是起源于脊索残余的罕见恶性肿瘤,多位于颅底至尾骨之间中线骨组织,大约45％位于骶尾骨,40％位于颅底,15％位于脊椎骨,位于桥脑前、鞍上和枕骨大孔区的硬膜内、腰髓硬膜外非骨源性脊索瘤也少有报道,作者报道一例下丘脑脊索瘤的少见病例。 患者,女,51岁,因严重头痛伴呕吐、意识状态进行性恶化5天来诊。外院曾CT检查示脑积水并给予了激素治疗,患者明显表现为偏头痛但未接受过偏头痛药物治疗,有吸烟史。入院体检:烦燥不安,言语不清,不能按嘱动作。视乳头水肿及脑膜刺激征 Chordoma is a rare malignant tumor originating from the chordal remnant. Mostly located in the midline bone between the base of the skull and the tailbone, about 45% are located in the sacrococcygeal bone, 40% are located in the skull base, 15% are located in the vertebra, Occipital foramen magnum area of ​​the epidural, spinal cord epidural non-osteoid chordoma is also rarely reported, the authors report a rare case of hypothalamic chordoma. Patient, female, 51 years old, diagnosed with severe headache with vomiting and progressive deterioration of consciousness for 5 days. Outside the hospital had CT examination showed hydrocephalus and given hormone therapy, the patient was obviously migraine but did not receive migraine drug treatment, a history of smoking. Admission medical examination: irritability, unclear speech, can not be asked to move. Papilledema and meningeal irritation